This editorial is an invited response to “Adherence to the 2014 American Academy of Pediatrics Palivizumab Prophylaxis Recommendations”

Rationale: Methicillin resistant Staphylococcus aureus (MRSA) is prevalent and consequential in cystic fibrosis (CF). Whole genome sequencing (WGS) could reveal genomic differences in MRSA associated with poorer outcomes or detect MRSA transmission. Objectives: To identify MRSA genes associated with low lung function and identify potential MRSA transmission in CF. Methods: We collected 97 MRSA isolates from 74 individuals with CF from 2017 and performed short-read WGS. We determined sequence type (ST) and the phylogenetic relationship between isolates. We aligned accessory genes from 25 reference genomes to genome assemblies. We classified the MRSA by accessory gene content and correlated the accessory genome to clinical outcomes. Results: The most prevalent ST were ST5 (N=55), ST105 (N=14), and ST8 (N=14). Closely related MRSA strains were shared by family members with CF, but rarely between unrelated individuals. Three distinct clusters of MRSA were identified by accessory genome content. The first included ST5 and ST105 strains and was common among older patients with lower FEV1. The second cluster included ST8, which was generally identified in younger patients. Sputum density of MRSA and Pseudomonas aeruginosa was higher in cultures from patients with ST5/ST105 compared to patients with ST8 at similar ages. Conclusions: In this CF cohort, we identified MRSA subtypes that predominate at different ages and differ by accessory gene content. ST5 and ST105 represented the most prevalent cluster of MRSA. ST8 MRSA was more common in younger patients and thus has the potential to rise in prevalence as these patients age.

Background: Asthma is a leading cause of pediatric hospitalization in the United States. Children hospitalized with asthma are often cared for in different care settings during a single hospitalization. Our objective was to study the reliability and safety of a new pediatric hospital-wide asthma severity score (HASS) across different care units within a single tertiary-care pediatric center. Methods. 150 patients between the ages of 2 and 18 years hospitalized with a principal diagnosis of status asthmaticus were included. Study patients were followed from initial triage in the emergency department until the time of medical readiness for discharge. Rates of medical errors, early transfers to a higher level of care and medically indicated hospital length of stay (LOS) were compared between 75 patients prior to and 75 patients after implementation of the HASS using retrospective chart review. Inter-rater reliability was determined by collecting independent HASS scores from blinded staff members after tandem or simultaneous patient assessment. Results. Inter-rater reliability among untrained staff members using the HASS was high. Rates of preventable adverse events and medical errors were low and not significantly different before and after implementation of the HASS. LOS was shorter after implementation of the HASS but without statistical significance. Rates of early transfer to a higher level of care were unchanged between study years. Conclusion. The HASS is a reliable asthma severity tool that can be used throughout hospitalization and also among multiple clinical providers to trend clinical progress and optimize communication, particularly during times of care handoffs.

Numerous studies in the past 10 years have reported on the neurocognitive sequalae of pediatric Sleep Disordered Breathing (SDB). Variations in criteria used to define SDB in conjunction with the wide variety of neuropsychological measures selected to evaluate cognitive consequences of SDB have resulted in discrepancies within the literature. This review summarizes the extant literature regarding cognitive effects of pediatric SDB across domains of global intelligence, attention, executive function, memory, language, and visuospatial ability. This review also addresses the proposed etiology underlying neurocognitive consequences of pediatric SDB. The differences in findings across the literature are highlighted and discussed throughout.

Introduction: Newborn screening (NBS) for cystic fibrosis (CF) was implemented in all US states and DC by 2010. This hypothesis generating study was designed to form the basis of additional research and to plan quality improvement initiatives. The aims were to describe the outcomes of infants with CF born during the first 9 years of universal NBS. Methods: We included participants in the CF Foundation Patient Registry born 2010-2018 with age at first CF event (first sweat test, clinic visit or hospitalization) by age 365 days. We assessed age of center-reported diagnosis, age at first CF event, demographics and outcomes for three consecutive 3-year cohorts born in 2010-2012, 2013-2015, and 2016-2018. Results: In 6354 infants, median age at diagnosis was earlier than median age at first CF event, which decreased from 1st cohort to 3rd cohort. Weight-for-age (WFA) was < 10th percentile in about 40% of infants at the first CF Center visit. Median WFA z-score at 1-2 years was > 0 but height-for-age (HFA) z-score was < 0 through age 5-6 years. The second cohort had a higher HFA z-score than the first cohort at age 5-6 years. Pseudomonas aeruginosa infection rates decreased over time. About 1/3 of infants were hospitalized in the first year of life across cohorts. Conclusion: Over 9 years of CF NBS, median age at first CF event decreased. CF NBS had positive health impacts but improving nutritional deficits and reducing infant hospitalizations remain targets for improvement.

Objective: Obstructive sleep apnea (OSA) is highly prevalent in children and requires an expensive and relatively unavailable sleep study for diagnosis. This study was undertaken to translate the previously validated OSA screening tool (POSAST) to the Thai language and assess its accuracy and test-retest reliability in at-risk symptomatic children. Study design: Prospective cross-sectional cohort study Methods: Pediatric patients clinically referred for suspected OSA who underwent overnight polysomnography (PSG) were recruited, and caregivers completed the Thai version of the POSAST. The same questionnaire was completed again after 2-4 weeks. Results: One hundred and ten subjects completed the study. The mean age was 8.4±2.9 years. The mean apnea-hypopnea index (AHI) was 10.9±11.9 events/hour. Test-retest reliability (Pearson correlation coefficient = 0.96, P<0.001) and internal consistency (Cronbach’s alpha coefficient = 0.82, P<0.001) between each question were excellent. A cumulative equation-derived score cut-off of 1.9 yielded 78.4% sensitivity and 50.0% specificity, while a numerical additive score cut-off of 8 corresponded to 81.1% sensitivity and 52.8% specificity for diagnosing moderate and severe OSA (AHI ≥5 events per hour) Conclusion: The internal consistency and reproducibility of the Thai version of the POSAST are satisfactory, display acceptable validity, and the instrument can be used for screening symptomatic Thai children for OSA.

Introduction: To help open the clinician dialogue regarding cannabis use in persons with CF in the U.S., we aimed to describe current practices of use assessment and documentation processes related to cannabis. Methods: A cross sectional, anonymous survey study was distributed via email to CF directors and coordinators and to the Cystic Fibrosis Foundation (CFF) listservs of nurse, pharmacist, dietitian, social worker and psychology care team members. The survey tool included multiple choice, scaled and open ended items, which assessed participants’ awareness of current cannabis laws in their state, prescribing practices for medical marijuana, screening and documentation practices, knowledge of and what indications participants believe cannabis and cannabidiol (CBD) could be beneficial. Data was analyzed using descriptive statistics. Results: There were 282 survey participants, with majority as providers (28%) and social workers (29%), representing all U.S. regions. Participants varied in terms of frequency of evaluating cannabis use, with 15.4% “always,” 48.4% “sometimes,” and 41% “rarely” or “never” asking about it. Regarding recreational versus medical cannabis use, 55.4% and 62.5% reported documentation of each type in the medical record, respectively. Participants reported appetite, pain, and nausea as the top three advocated indications for use. About 35% and 72% of participants felt “slightly” or “not at all” prepared to answer patient/family questions about cannabis and CBD, respectively. Conclusions: The approach to cannabis use assessment, documentation, and education across CF care centers is variable. There is a need for care team and patient/caregiver education materials about cannabis/CBD and CF.

Abstract Aim: To evaluate the respiratory functions of children with nephrotic syndrome (NS) by IOS and its correlation with spirometry. Methods: Fifty-five NS patients aged 3–18 years were included as the study group and 40 healthy children of the same age formed the control group. Patients were divided into nephrotic phase (first attack and relapse) and remission. Demographic, anthropometric and laboratory data of the children were recorded. Respiratory functions were evaluated by IOS and spirometry. Children over 6 years old performed both IOS and spirometry while children under 6 years performed only IOS. Results: The R (R5%, R10%, R5-20) and AX and Z5% values of IOS in patients with nephrotic phase were higher than remission patients and control group while spirometry indices of FEV1%, FEV1/FVC, PEF% and MEF25-75% were lower. FEV1% showed negative correlation with R5%, R10%, R15%, X10% and X15% results, FEV1/FVC% showed negative correlation with R5%, R5-20 and X15% results, FVC showed negative correlation with X10% results, MEF25-75% showed negative correlation with, R5%, R10%, R5-20, X15%, F res, Z5% and AX results. Conclusion: Our study demonstrated that respiratory functions measured by IOS and spirometry were affected at the time of nephrotic phase in NS patients. And IOS, a novel method easily applicable even in small children, is a valuable and reliable tool to detect this condition; given its good correlation with spirometry. Keywords Nephrotic syndrome, Respiratory functions, Spirometry, Impulse Oscillometry

Disseminated tuberculosis (TB) in the pediatric population is relatively rare in the United States with variable nonspecific presentations. In this letter we discuss the case of 2-year-old child with a lung mass and central neurogenic hyperventilation with primary respiratory alkalosis as a rare pediatric presentation of disseminated tuberculosis with TB meningitis and pulmonary tuberculosis.

Introduction: With improving mortality rates in pediatric acute respiratory distress syndrome (PARDS), functional outcomes in survivors are increasingly important. We aim to describe the change in functional status score (FSS) from baseline to discharge and to identify risk factors associated with poor functional outcomes. Methods: We examined clinical records of patients with PARDS admitted to our pediatric intensive care unit (PICU) from 2009 to 2016. Our primary outcome was acquired morbidity at PICU and hospital discharge (defined by an increase in FSS ≥3 points above baseline). We included severity of illness scores and severity of PARDS in our bivariate analysis for risk factors for acquired morbidity. Results: There were 181 patients with PARDS, of which 90 (49.7%) survived. Median pediatric index of mortality 2 score was 4.05 (1.22, 8.70) and 21 (26.6%) patients had severe PARDS. 59 (65.6%) and 14 (15.6%) patients had acquired morbidity at PICU and hospital discharge, respectively. Median baseline FSS was 6.00 (6.00, 6.25), which increased to 11.00 (8.75, 12.00) at PICU discharge before decreasing to 7.50 (6.00, 9.25) at hospital discharge. All patients had significantly higher median FSS score at both PICU and hospital discharge compared to baseline. Feeding and respiratory were the most affected domains. After adjusting for severity of illness, severity categories of PARDS was not a risk factor for acquired morbidity. Conclusion: Acquired morbidity in respiratory and feeding domains was common in PARDS survivors. Specific attention should be given to these two domains of functional outcomes in these children.

This is a case report describing a rare condition.A 12-year-old boy had a blood cast of the left bronchial tree from bronchial hemorrhage caused by respiratory foreign body.He underwent rigid bronchoscopy and cast removal.

Bronchopulmonary dysplasia is a relatively common and severe complication of prematurity, and its pathogenesis remains ambiguous. Revolutionary advances in microbiological analysis techniques, together with the growing sophistication of the gut-lung axis hypothesis, have resulted in more studies linking gut microbiota dysbiosis to the occurrence and development of bronchopulmonary dysplasia. The present article builds on current findings to examine the intrinsic associations between gut microbiota and bronchopulmonary dysplasia. The gut microbiota affects bronchopulmonary dysplasia via several potential mechanisms including alteration of the gut-lung axis, promotion of inflammation and the ensuing growth effects, therefore these are also investigated. By evaluating the potential mechanisms, new therapeutic targets and potential therapeutic modalities for BPD can be identified from a microecological perspective.

Bullying-induced dyspnoea in children: a case seriesIan P. Sinha 1,2Claire Hepworth 1Sujata De 1Sunil D. Sharma 1Ian Street 1Philip J. Lawrence 1Thomas Hampton* 1,21 Alder Hey Children’s Hospital, Liverpool, UK2 Division of Child Health, University of Liverpool, UK*Corresponding author ([email protected])Dear EditorWe conduct a multidisciplinary complex breathlessness clinic for children1. We conduct spirometry before and after a treadmill exercise test (until the child is breathless), continuous nasal laryngoscopy, pulse oximetry, and calculation of maximal oxygen consumption (VO2max). Here we describe a series of children who presented with troublesome breathlessness that appeared to be caused, or exacerbated by, being bullied.Case 1 (14 year old white female): She was a highly competitive sportsperson, but was recently unable to train or compete. She described her breathlessness as ‘air getting stuck in her throat’, and had a non-specific cough. She had frequent admissions to hospital, treated as presumed asthma attacks. The referring clinician felt her asthma was of insufficient severity to cause her problems. She had no documented obstruction on spirometry in clinic, nor and Fractional Exhaled Nitric Oxide (FeNO) was normal. In clinic she managed a few minutes of running, before suddenly stopping. There was no evidence of exercise-induced bronchoconstriction or exercise-induced laryngeal obstruction (EILO), but she had features of dysfunctional breathing (DB). On questioning she described feeling bullied by parents of other children in her sports team, whom she described as overcritical and disparaging. She was taught breathing exercise and referred to psychological services. Her symptoms and asthma attacks improved, and she recommenced competitive sports.Case 2 (10 year old white female): She had a chronic wet cough since the age of six months. She underwent flexible bronchoscopy which identified mild tracheobronchomalacia. She recently described breathlessness on mild exertion that was disproportionate to the degree of tracheobronchomalacia. Physiological testing never demonstrated airways obstruction, or raised FeNO. In clinic she started running but stopped within minutes, with no physiological evidence of increased work of breathing or bronchoconstriction. Laryngoscopy was normal. On questioning she described that she was bullied at school. Specifically, she described that children would not sit near her because of her cough. She was followed up in respiratory physiotherapy clinic, and her symptoms and exercise tolerance improved.Case 3 (13 year old white female): She had no medical diagnoses at the time of testing, but previously had tonsillectomy because of recurrent tonsillitis. She described breathlessness on exertion. She stopped running very suddenly, as soon as she felt breathless on the treadmill. The breathlessness started as discomfort underneath her ribs and in her throat. Spirometry and laryngoscopy were normal before and after exercise. She had apical breathing and hyperventilation at rest, suggestive of dysfunctional breathing. She described being bullied at school. She did not attend follow up sessions with physiotherapy and was discharged from the service.Case 4 (9 year old black male): He was treated for mild asthma which had, until recently, been well controlled. He had become withdrawn, and was not enjoying playing sports despite previously being very athletic. On questioning he described suffering significant and long-standing racial bullying at school. He discussed this with the teachers but his symptoms seemed to develop after he felt like his complaints were not taken seriously. His breathlessness and exercise tolerance improved temporarily after enrolling in a community sports program for children with asthma 2, and he was much better after moving school.Across these cases, we identified common themes:The character and severity of the breathlessness was out of keeping with their underlying diagnoses, and was intensely unpleasant. All children described non-specific and variable symptoms of pain in their abdomen, joints, or chest.They appeared withdrawn, unhappy, and lacking in self-confidence. They had slouched posture, and spoke quietly.They had very sudden and surprising termination of exercise after starting to feel breathless, with no significant physiological evidence of increased work of breathing – we noticed a stark difference compared with other children who saw breathlessness as a challenge and would continue to run long after showing signs of tachypnoea and tachycardia.In all cases, it was the healthcare professionals who raised the subject of bullying. The children had felt that they had raised concerns about being bullied but felt these were dismissed.When children engaged with physiotherapy, we noticed improvements. The children were relieved when we did not find significant anatomical or physiological diagnoses, and all agreed their breathlessness was caused by bullying.We feel that these patterns relate to a phenotype of childhood dyspnoea specifically related to bullying. Anxiety is associated with tachypnoea, but in our experience the pattern of breathing in these children was different: their shallow, rapid breathing at rest was felt to be related to a slouched posture causing restriction, and all had a sudden cessation to exercise after very minimal exertion. We suggest these are manifestations, specifically, of low self-esteem. It was notable that they described the breathlessness as intensely uncomfortable. The neural pathways involved in driving respiration, and sensing breathlessness, are complex, and we postulate that they are affected by low self-esteem.This, to our knowledge, is the first description of bullying-induced dyspnoea in children, as a separate phenomenon to anxiety-related hyperventilation. There may be crossover with other psychological, physiological and anatomical problems, and further research is warranted. Having asthma is a risk factor for being bullied3, and bullying is associated with worse asthma control4. A recent review has also identified possible associations between bullying and the development of childhood asthma5. It is important ask about bullying when taking a history from a child with unexplained or disproportionate breathlessness.

Objective: Description of the use of corticosteroids for the management of parapneumonic pleural effusion in children. Methods: Retrospective monocenter cohort study of all children hospitalized with a discharge diagnosis of parapneumonic pleural effusion during a 15-year period. Results: We documented 97 cases of parapneumonic effusion during the study period, with a median age (interquartile range (IQR)) of 43 (33-61) months. Most of the children benefited from an evacuation of the pleural effusion (89/97, 91.8%): 21 patients (21.6%) were treated with needle thoracocentesis only, while a chest tube was inserted in 68 children (70.1%). Thirty-two patients (33%) were treated with intrapleural fibrinolysis. Fifty-five children (56.7%) received corticosteroids for persistent fever. The median time (IQR) between hospital admission and initiation of corticosteroids was 5.5 (4-7) days. When corticosteroids were initiated, children were febrile since 9 (IQR 8-11) days. The fever ceased in a median (IQR) of 0 (0-1) day after corticosteroids initiation. Only 1 patient required a video-assisted thoracoscopy that was provided because of morphological reasons (morbid obesity). No children treated with corticosteroids required surgery. All children were discharged alive from hospital. The median (IQR) hospital length of stay was 11 (8-14) days, with no difference between children with and without corticosteroids. Conclusion: Our results indicate that corticosteroids could be associated with a significant reduction in the use of surgical procedures and with a prompt clinical improvement. Corticosteroids could thus offer a non-invasive therapeutic alternative for children with parapnemonic effusions when antibiotics and pleural drainage are considered a failure.

Cysticercosis in humans is a serious public health problem, predominantly affecting low and middle-income countries (LMICs). Cysticercosis, the infection with the larval form of the pork tapeworm, Taenia Solium has high prevalence in areas where there are poor sanitary conditions and domestic pig without adequate veterinary control. Humans are the definitive host and pigs are the main source of infection. Human infection occurs when pork is eaten raw or undercooked. Ingested eggs or proglottids hatch into larvae form, which penetrate the intestinal wall into the blood stream and migrate into different organs including subcutaneous tissues, brain, eyes and rarely heart or lung, where they mature into cysticerci. Pulmonary cysticercosis has been rarely described; case reports are predominantly in adults and are usually of disseminated disease. In children the data are very scarce, with a single case report of a two-year old child with pulmonary infiltration, eosinophilia and subcutaneous cysticercosis.

Acute viral bronchiolitis (AVB) is the most common infection of the lower airways in children under 2 years of age. Attempts to determine the severity of the disease based on clinical and radiological manifestations are a major challenge. Measurements of the anatomy of the trachea, main bronchi and bronchioles are not limited to pure anthropometry, but are also useful for better knowledge and applicability in pulmonary physiology, thoracic surgery, anesthesiology and in the care of critically ill patients. This is a cross-sectional, retrospective study, which included all patients admitted to a tertiary hospital in the city of Porto Alegre, over a period of one year, with a diagnosis of AVB. The interbronchial angle (ITB) of these patients was measured and clinical and epidemiological characteristics were analyzed. A total of 425 patients were included, diagnosed with BVA by respiratory syncytial virus (RSV) confirmed by immunofluorescence. Most of these patients were male (59.5%) and the median age was 130 days, 91.11% of them required oxygen therapy through a nasal catheter, 3.3% used non-invasive ventilation (NIV) and 4% mechanical ventilation (MV) . Among the studied patients, we obtained only one death (0.2%). Those who required MV or NIV and support in an intensive care unit were considered serious. The mean ITB was lower for these patients than for less severe ones. It is concluded that the ABI has a correlation with the prognosis of patients with AVB and, after further studies, can be used as a severity score.

Pulmonary physiology is a core element of pediatric pulmonology care and research. This article reviews some of the notable publications in physiology that were published in Pediatric Pulmonology in 2020.

Objectives: Pneumothorax (PTX) in newborns is a life-threatening condition associated with high morbidity and mortality especially in premature infants. The frequency of PTX in neonates at different gestational ages (GA) and its impact on neonatal mortality have not been quantified. We aimed to determine: 1) the prevalence of PTX in neonates at different GA from ≤24 weeks to ≥37 weeks, 2) the impact of PTX on mortality per GA, and 3) the impact of PTX on the length of stay (LOS) per GA. Methods: The national Kids’ Inpatient Database (KID) for the years of 2006 to 2012 were used. We included all infants admitted to the hospital with a documented GA and ICD9 code of pneumothorax. Bivariate and multivariate analyses were conducted and odds ratios (OR) were calculated. Results: A total of 10 625 036 infants were included; of them 3665 infants (0.034 %) had a diagnosis of PTX, with highest prevalence at ≤24 weeks GA (0.67%), and lowest at term (0.02%). The overall mortality rate of patients with PTX was 8.8%, and greater in preterm (16.3%) vs. term infants (2.7%). The association of mortality with PTX was greatest at GA of 29−32 weeks (OR = 8.55 (95% CI: 6.56−11.13). Infants who survived until discharge had a median of 2–12 days longer length of stay depending on GA category. Conclusions: The prevalence of PTX peaks in infants <24 weeks, however its impact on mortality is greatest at 29-32 weeks. PTX is associated with longer length of stay in survivors.